Scleroderma

Scleroderma or systemic sclerosis is a multisystemic disease, pertaining to the category of collagenosis; it is manifested by a pathological proliferation of the collagen cells in the body, which together with other extracellular proteins - are deposited in excess in teguments and tissues. These processes will lead to fibrosis of the teguments, of the blood vessels, of the gastro-intestinal tract, of the heart, of the lungs and of the kidneys. There are also rheumatic manifestations, such as inflammation of the organs of the locomotor and cardio-vascular apparatus, accompanied by articular pains.

Scleroderma was classified into: localized and generalized scleroderma, depending on how deeply the tegument and the internal organs are affected. In the localized forms, the fibrosis process involves only the tegument, the muscles and the tissues in their close proximity. It does not affect the internal organs, but the skin loses its integrity in time and the lesions become permanent. There can be distinguished to clinical subforms of the localized scleroderma:

• morphea, manifested by spare dermatoses on the fingers, on the arms, on the forearms and lower limbs. Skin loses its elasticity, it grows pigmented; it becomes hardened and thick
• linear scleroderma is localized in one of the lower limbs; is a strap of linear hardened hyperpigmented tegument In children, the sclerodermic process may arrest even the growth of the bone in the affected limb

In the generalized forms, scleroderma starts by an inflammatory process, followed by the induration of the tegumentary tissue on the limbs, trunk and face. Tissue fibrosis also affects the esophagus, the intestines, the lungs, the heart and the kidneys. The pathological alterations of the cardio-vascular system pose the risk of hypertension and cerebral vascular accident. The patients with generalized or systemic scleroderma show the symptoms of the CREST syndrome:

• calcinosis
• tegumentary indurations
• telangiectasis
• rheumatic symptoms: arthritis
• myopathia
• esophageal disorders: disorders in the muscle functions, and disorders of the esophageal tube, burning in the esophagus and in the chest
• pulmonary fibrosis
• congestive cardiac insuficiency
• renal dysfunctions
• Raynaud syndrome symptoms: spasms in the capillaries that vascularise the fingers and the toes, the nose, the tongue and the ears; the areas inadequately vascularized change their color into blue, white and then into red when they are exposed to very low or very high temperatures, or when the patient undergoes very powerful emotions.

Causes and treatment

The pathological process of collagen proliferation is caused by a dysfunction in the production of this protein. The production of collagen is done with the participation of the thesaurus organs, except the heart, namely the liver, the lungs, the pancreas, the spleen and the kidneys. The disorders in collagen production occur when the areas carrying out these functions in the respective organs become non functional or work at low capacity, as a result of a poor innervation and of a vascularisation too rich but with a low oxygen supply. Therefore, treatment of scleroderma involves a complex approach, because all the thesaurus organs must be treated simultaneously in order to break the vicious circle of the disease. As an area in each organ is regenerated, a symptom disappears, similarly to the domino effect, until all the manifestations are completely eliminated. When the thesaurus organs restore their function within optimum parameters and the collagen production is normalized, the disease is considered to be cured.

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